Intracranial Dural Arteriovenous Fistulas with Cortical Venous Drainage: Radiosurgery as an Effective Alternative Treatment.

OBJECTIVE: To evaluate the clinical and radiological outcome of Gamma Knife radiosurgery (GKS) in treatment of intracranial dural arteriovenous fistula (DAVF) with cortical venous drainage (CVD) and compare it with the outcome of endovascular therapy. METHODS: Patients who underwent GKS or endovascular therapy for intracranial DAVF with CVD over 10 years (January 2007 to December 2016) at the All India Institute of Medical Sciences, New Delhi, were included. Demographics, clinical presentation, imaging details, and follow-up clinical status were reviewed retrospectively. Clinical follow-up was conducted once every 6 months. Radiological follow-up using digital subtraction angiography was performed at a mean 24 months after intervention. Patients with clinical follow-up of <1 year were excluded from the study. RESULTS: The study included 35 patients (26 in embolization group and 9 in GKS group) who had intracranial DAVF with CVD were included in the study. Clinical improvement was seen in 77.78% of the patients who received GKS and 57.7% of the patients who underwent embolization (P = 0.431). Complete obliteration of DAVF was seen in 55.56% of the patients in the GKS group and 57.7% of the patients in the embolization group (P = 1). GKS was at least as effective as embolization in terms of clinical and radiological outcome in treatment of intracranial DAVF with CVD. CONCLUSIONS: Contrary to popular perception, GKS should be considered as an effective first-line treatment alternative of intracranial DAVF with CVD.

Fourth ventricular hamartoma presenting with status epilepticus treated with emergency surgery in an infant.

A 4-week-old infant presented with hemifacial spasms noticed from the 4th day after delivery. These progressed in severity, with generalization every 3-4 h. On admission, the infant went into refractory status epilepticus and had to be electively ventilated and taken for surgery on a semi-emergency basis. MRI showed a fourth ventricular hamartoma and video EEG showed spikes that were synchronous with the facial 'twitches' with generalization. Following the first surgery, the infant had an initial complete recovery, but developed recurrence of facial twitches after 2 weeks. Repeat MRI showed a small residual tumor which was re-operated and completely excised (at 8 weeks). Following this, the patient had complete recovery from seizures (5-year follow-up). This is the youngest patient reported presenting with status epilepticus with a fourth ventricular hamartoma operated successfully.

Juvenile pilocytic astrocytoma presenting with subarachnoid hemorrhage. Case report and review of the literature.

The authors report a case of a 13-year-old boy with juvenile pilocytic astrocytoma (JPA) presenting with subarachnoid hemorrhage (SAH). The patient experienced sudden onset of headache, vomiting, and loss of consciousness. Cranial computerized tomography scanning revealed blood within basal cisterns and the third ventricle. Angiography demonstrated normal cerebral vasculature and upward displacement of the bilateral A, segments of the anterior cerebral artery. Magnetic resonance (MR) imaging revealed a chiasmatic/hypothalamic mass with evidence of hemorrhage. The mass was surgically decompressed. Histopathological examination showed evidence of JPA. In all cases of SAH in which there is blood around the third ventricle and a raised A1 segment on angiography, MR imaging should be performed. The presence of a normal sella turcica, as well as indistinct margins between the tumor and the opticochiasmatic apparatus should raise suspicion about the lesion.

Distraction, compression, and extension reduction of basilar invagination and atlantoaxial dislocation: a novel pilot technique.

BACKGROUND: The management of basilar invagination (BI) and atlantoaxial dislocation (AAD) is a challenge. OBJECTIVE: To describe a new innovative method to reduce BI and AAD through a single-stage posterior approach. METHODS: Thirty-five patients had irreducible BI and AAD (May 2010 to April 2012). In all patients, reduction of AAD and BI was achieved by using an innovative method of distraction and spacer placement, followed by compression and extension. A C1 lateral mass/C2 translaminar screw was performed in cases where the C1 arch was not assimilated, and occipito-C2 translaminar screw fixation was performed in cases where the C1 arch was assimilated. RESULTS: Thirty-two of 35 (94%) patients improved clinically and 2 patients had stable symptoms (mean Nurick postoperative score = 1.4; preoperative score = 3.7). AAD reduced completely in 33/35 patients and >50% in 2. BI improved significantly in all patients. Solid bone fusion was demonstrated in 24 patients with at least 1-year follow-up (range, 12-39 months; mean, 19.75 + 7.09 months). The duration of surgery was 80 to 190 minutes, and blood loss was 90 to 500 mL (mean, 170 ± 35 mL). There was 1 death because of cardiac etiology and 1 morbidity (wound infection). CONCLUSION: Distractive compressive extension and reduction of BI and AAD seems to be an effective and safe method of treatment. It is different from the earlier described techniques, because it is the first procedure that uses a spacer not, only for distraction, but also as a pivot to perform extension to reduce the AAD.

Middle meningeal artery arising from the basilar artery: report of a case and its probable embryological mechanism.

An extremely rare variation of the (left) middle meningeal artery (MMA) originating from the basilar artery, detected incidentally during cerebral angiography, is reported. The right MMA was normal and an accessory meningeal artery arising from the maxillary artery was present on both the sides. The foramen spinosum on the variant side was absent. This abnormal origin of the MMA can be explained by the presence of a perineural arterial network in the region of the Gasserian ganglion, formed by branches of the developing basilar and stapedial arterial systems; the middle meningeal-basilar arterial channel opening up in the absence of a normally developing MMA.

Treatment of vertebral hemangiomas with absolute alcohol (ethanol) embolization, cord decompression, and single level instrumentation: a pilot study.

BACKGROUND: Vertebral hemangiomas (VH) are the most common lesions of the vertebral column. OBJECT: To evaluate the role of intraoperative ethanol embolization, surgical decompression, and instrumented fusion in VH presenting with myelopathy. METHODS: This is was a prospective study of single-level symptomatic VH with cord compression. Exclusions were as follows: pathological fractures, deformity, or multilevel pathologies. Surgery consisted of intraoperative bilateral pedicular absolute alcohol injection and laminectomy at the level of pathology followed by a short-segment instrumented fusion using pedicle screws. RESULTS: Ten patients (mean, 26.8 ± 18.11; range, 10-68 years; 8 females) were treated with use of this technique. Clinical features included myelopathy with motor and sensory involvement in all (4 paraplegic), sphincter involvement (8), and severe local pain (5). The preoperative American Spinal Injury Association (ASIA) scores were A (3), B (1), and C (6). All had pan vertebral body VH with severe cord compression. The mean surgical time was 102 ± 22 minutes; average blood, 296 ± 90.82 mL. Mean amount of absolute alcohol injected was 12.6 ± 4.7 mL (1 requiring 25 mL). Immediate embolization was achieved in all patients allowing laminectomy and soft-tissue hemangioma removal. Postsurgery, all patients showed improvement (sphincters improved in 4) at a follow-up ranging 12 to 26 months (transient neurological deterioration in 1). Postsurgery ASIA scores were D (5) and E (5) at last follow-up. Two patients showed evidence of bone sclerosis on follow-up CT scans at 1.2 and 1.5 years. CONCLUSION: This procedure seems to be a safe, efficient method to treat VH with severe cord compression. It seems to serve the purpose of providing embolization, cord decompression, and rigid fusion at the same sitting.

Association of craniovertebral and upper cervical anomalies with dermoid and epidermoid cysts: report of four cases.

OBJECTIVE AND IMPORTANCE: Four patients with craniovertebral junction anomalies (CVJ) and Klippel-Feil cervical fusion defects associated with dermoid and epidermoid cysts are described. CLINICAL PRESENTATION: During the 10-year period from 1994 until May 2004, 435 patients with developmental CVJ anomalies presented to our institution. Four of these patients harbored a constellation of CVJ anomalies with dermoid and epidermoid cysts (hospital prevalence, 0.9%). All patients (ages 18, 23, and 25 yr) presented with features of spastic quadriparesis, restriction of neck movements, and raised intracranial pressure. Magnetic resonance imaging showed features of CVJ anomalies in all patients (occipitalization of C1, 3 patients; basilar invagination, 3 patients; atlantoaxial dislocation, 4 patients; and an abnormal posteriorly pointed dens, 1 patient), along with a Klippel-Feil anomaly (Patients 1-3, 2nd and 3rd cervical vertebrae). Patient 4 also had a Chiari malformation with syrinx. In addition, all four patients had coexisting dermoid or epidermoid cysts (Patients 1 and 3, midline posterior fossa epidermoid; Patient 2, midline posterior fossa dermoid; Patient 4, quadrigeminal cistern epidermoid). INTERVENTION: Patients 1 and 2 underwent a posterior midline approach, excision of the tumor, and an occipitocervical fusion (the atlantoaxial dislocation was reducible). Patient 3 underwent transoral excision of the odontoid, followed by tumor excision and occipitocervical fusion via the posterior route. These patients had uneventful recovery. Patient 4 did not undergo an operation. CONCLUSION: Association of CVJ anomalies with Klippel-Feil and dermoid and epidermoid cysts is extremely rare, with only two previously reported cases. The patient who presented with a quadrigeminal cistern epidermoid with a craniovertebral junction anomaly and Chiari malformation with syrinx is the first such case ever reported in the literature. Apart from therapeutic implications, these patients may shed new light on the embryogenesis. Furthermore, the gathering of these manifestations may constitute components of a new syndrome that has gone unnoticed until now.

Bipartite atlas with os odontoideum: case report.

STUDY DESIGN: A case report of bipartite atlas associated with os odontoideum and review of the pertinent literature are presented. OBJECTIVE: To illustrate an unusual association of bipartite atlas and os odontoideum and explain the embryological basis. SUMMARY OF BACKGROUND DATA: To the authors' knowledge, only one case of bipartite atlas with os odontoideum had been reported previously. Most of the previously reported cases of bipartite atlas are asymptomatic. METHODS: A 16-year-old boy presented with a 2-month history of weakness and numbness of all four limbs after sustaining a minor head trauma. Radiographs of cervical spine revealed aplasia of anterior arch of atlas, ventral displacement of C1 over C2 on flexion, which reduced on extension. CT scan showed anterior arch aplasia, posterior arch midline defect, and os odontoideum, which had a small projection on the anterior surface at the level of anterior arch. MRI demonstrated increased cord signal at C1-C2 levels on T2-weighted image. Transoral odontoidectomy and posterior fixation of occiput with C2-C3 spinous processes was performed. RESULTS: The patient had significant improvement over next 3 months. CONCLUSIONS: We described a rare association of an anterior arch aplasia, posterior arch defect and os odontoideum. The natural history of patients with os odontoideum suggests that these people have a potentially precarious existence.